Systemic Lupus Erythematosus: The Great Imitator

butterfly or malar rash in SLE

Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can affect any part of the body, hence the term systemic. Most commonly affected organs are the joints, skin, heart, lungs, kidneys, liver, blood vessels, and nervous system. In 2008, around 250.000 Americans have SLE. Women aged 20-30 years old are nine time more likely to be affected than men. African American and Asians are more commonly affected and the disease tend to be more severe in these ethnic groups.

Diagnosis

As in any autoimmune disorder, the immune system lost the ability to differentiate self and non self, producing auto antibody that attacks normal cells. Why our immune system turned against us is still unknown, but viral infection, sunlight and allergies may trigger the changes.

SLE is dubbed the great imitator because the symptoms can mimic other diseases, making the diagnosis a challenging one to make. The course of disease is completely unpredictable with flare-ups alternating with remissions. Severity ranges from mild to life threatening. Initially, most patients unspecific symptoms like fatigue, rashes, painful joints, and low grade fever.

Based on The American College of Rheumatology's criteria, a person is said to have SLE if he or she has at least four of the symptoms listed below.

the location of rashes in SLE

• Butterfly shaped rashes over the cheeks (malar rash), oval shaped rashes with raised borders (discoid rash), or rash over sun exposed skin
• Arthitis in two or more joints lasting more than a few weeks
• Chest pain when taking deep breaths, a sign of inflammation of the outer linings of the lung (pleuritis) or the heart (pericarditis)
• Kidney problems ranging from mild protein or blood in the urine to renal failure
• Nervous system disturbances like seizures, stroke, or delirium
• Sores in the mouth
• Abnormal lab results: 
• low red blood cells, low white blood cells, or low platelets
• positive auto antibodies like anti nuclear antibody (ANA) or anti double stranded DNA (anti-dsDNA), anti Smith (referred to as anti-Sm) or antiphospholipid antibodies, or a false positive test for syphilis

Treatment

Sadly there is no cure for SLE and its management can be rather complicated. The good news is that immunosuppresive therapy can greatly improve survival. Maintaining a good quality of life remains the greater challenge, however.

The medications commonly used for SLE includes:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce joint pain and inflammation.
• Antimalarial drugs such as hydroxychloroquine help with fatigue, rashes, joint pain, and mouth sores.
• Immunosuppressants such as azathioprine, cyclophosphamide, cyclosporin are used in patients with more severe and wide-spread symptoms.
• Corticosteroids is also used in the more severe form of SLE.
• Biological agents such as rituximab and abatacept modulate the faulty immune system.

The Basics of Autoimmune Diseases

The immune defense system is designed to defend our body from foreign substance and other harmful invaders. A healthy immune system has the ability to differentiate between self and non-self, that is knowing what to kill and what to leave alone. In autoimmune diseases, the immune system lost the ability to tell the difference and attacks normal cells by mistake.

There are more than 80 different types of autoimmune diseases affecting more than 23.5 million people in the United States, causing disability due to the chronic nature of these disorders. In general, women especially the ones of African American, Hispanics, and Native American racial background have higher risks.

What happens in autoimmune diseases?

An antigen is a substance that has the ability to trigger the immune system. It can be bacteria, virus, fungi, blood or tissue from another person or species, and toxin. When an antigen entered our body, white blood cells produces antibodies to destroy it. There is also a type of white blood cells called the T cells that keeps antibody production in check and make sure it doesn't attack what it's not supposed to. In autoimmune diseases, the antibody turned to attack our own cells and is called auto antibody.

What causes the changes in our immune system behavior resulting in autoimmune diseases is unknown. Some have proposed that the antigen itself triggers this conversion. A similarity between the bacteria or virus genes with our own may confuses our immune system.

An autoimmune disease can target any organ in our body. It can also affect more than one bodily systems at the same time. Blood vessels, red blood cells, glands, joints, muscles, connective tissues, and the skin are commonly affected. Some examples of autoimmune diseases are systemic lupus erythematosus, multiple sclerosis, type 1 diabetes, rheumatoid arthritis, and Graves' disease.

Symptoms

Diagnosing autoimmune diseases provides one the most difficult challenges for physician because the symptoms varies greatly, may come and go, and can mimic other diseases. For most people, the initial symptoms are quite vague: fatigue, muscle aches, low grade fever, and general unwell feeling. Over time, symptoms will develop depending on affected organs.

Management

It's difficult to cure autoimmune diseases because the exact cause is unknown in the first place, but symptoms can be reduced and complications prevented. Treatment plan depends on the specific diseases. Often times, if the symptoms gets worse, immunosuppressive drugs may be needed. These drugs weakens your immune system and infection can be a problem for people who uses them for long terms.

Understanding Allergy

What is allergy?

Allergy occurs when your immune system reacts against a foreign substance. An allergen is a substance that can trigger an allergic reaction. When you're exposed for the first time, your immune system will think that something harmful has entered your body. It then produces antibodies that 'remembers' that particular allergen. The next time you're exposed to the same allergen, your body will react by producing more antibodies and releasing chemicals that cause allergy symptoms.

What usually triggers allergy?

Pollen and insect stings are common allergy trigger

Common allergy triggers include:

• Airborne allergens: pollen, pet dander, dust mites and mold
• Foods: peanuts, eggs and milk, wheat, soy,  fish, shellfish, shrimp
• Medications: penicillin or penicillin-based antibiotics, sulfa drugs 
• Insect stings
• Latex 

What are allergy symptoms?

Symptoms varies depending on how you came into contact with the allergen. The allergic reaction may involve the airways, sinuses and nasal passages, skin, and digestive system. For example, you may experience runny nose and sneezing if you breathe in pollen and skin rashes if you came into contact with house dust mites. Severity of symptoms ranges from mild to life threatening such as in the case of anaphylaxis.

There are many forms of allergy:

• Hay fever or allergic rhinitis. This is a type of allergy towards inhaled allergen. It causes nasal congestion, runny nose, and conjunctivitis.
• Atopic dermatitis or eczema. Affects your skin causing rashes, itchiness, and flaking.
• Food allergy. Causes swelling of the lips, tongue, face, or throat, tingling sensation in your mouth, or diarrhea.
• Anaphylaxis. This is a life threatening medical emergency. Allergy towards foods, drugs and insect stings have the potential to trigger anaphylaxis. Symptoms of anaphylaxis include lightheadedness to loss of consciousness, rapid weak pulses, skin rash, airway swelling, nausea and vomiting. If you experience any of these symptoms, immediately call an emergency number or 911 for medical assistance or inject yourself with emergency epinephrine.

 

What about treatment?

Allergy managements include avoiding allergen, taking medications to relieve symptoms (antihistamines, decongestant, corticosteroids, cromolyn), and immunotherapy.